Pharmacological therapy for cystic fibrosis: from bench to bedside (Q37887169)

24 August 2017

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title

Pharmacological therapy for cystic fibrosis: from bench to bedside (English)

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24 August 2017

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5

Olga Zegarra-Moran

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24 August 2017

Frédéric Becq

1

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24 August 2017

Marcus A Mall

series ordinal

2

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24 August 2017

David N Sheppard

series ordinal

3

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24 August 2017

Massimo Conese

series ordinal

4

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24 August 2017

language of work or name

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1 June 2011

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Journal of Cystic Fibrosis

24 August 2017

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24 August 2017

10 Suppl 2

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24 August 2017

S129-45

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Cystic fibrosis since 1938.

24 August 2017

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https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Cystic fibrosis mortality and survival in the UK: 1947-2003

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Structure and function of the CFTR chloride channel

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

CFTR is a conductance regulator as well as a chloride channel

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

1 reference

7 January 2021

Gene and cell therapy for cystic fibrosis: from bench to bedside

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Cystic fibrosis: genotypic and phenotypic variations

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Processing of CFTR: traversing the cellular maze--how much CFTR needs to go through to avoid cystic fibrosis?

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Gating of the CFTR Cl- channel by ATP-driven nucleotide-binding domain dimerisation

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Mutations in CFTR associated with mild-disease-form Cl- channels with altered pore properties.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Multi-ion pore behaviour in the CFTR chloride channel

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Mechanism of dysfunction of two nucleotide binding domain mutations in cystic fibrosis transmembrane conductance regulator that are associated with pancreatic sufficiency.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Localization of cystic fibrosis transmembrane conductance regulator in chloride secretory epithelia

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

PTC124 targets genetic disorders caused by nonsense mutations

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Rescue of functional delF508-CFTR channels in cystic fibrosis epithelial cells by the alpha-glucosidase inhibitor miglustat

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Parallel improvement of sodium and chloride transport defects by miglustat (n-butyldeoxynojyrimicin) in cystic fibrosis epithelial cells.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Influence of cell background on pharmacological rescue of mutant CFTR.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Specific rescue of cystic fibrosis transmembrane conductance regulator processing mutants using pharmacological chaperones

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Phosphatase inhibitors activate normal and defective CFTR chloride channels

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Actions of genistein on cystic fibrosis transmembrane conductance regulator channel gating. Evidence for two binding sites with opposite effects

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Molecular pharmacology of the CFTR Cl- channel

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

On the mechanism of MgATP-dependent gating of CFTR Cl- channels

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

CFTR channel opening by ATP-driven tight dimerization of its nucleotide-binding domains

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

The First Nucleotide Binding Domain of Cystic Fibrosis Transmembrane Conductance Regulator Is a Site of Stable Nucleotide Interaction, whereas the Second Is a Site of Rapid Turnover

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Capsaicin potentiates wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride-channel currents

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Binding site of activators of the cystic fibrosis transmembrane conductance regulator in the nucleotide binding domains.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Functional analysis of mutations in the putative binding site for cystic fibrosis transmembrane conductance regulator potentiators. Interaction between activation and inhibition

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Role of the amiloride-sensitive epithelial Na+ channel in the pathogenesis and as a therapeutic target for cystic fibrosis lung disease

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

TMEM16A protein: a new identity for Ca(2+)-dependent Cl⁻ channels

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Pharmacology of CFTR chloride channel activity.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

CFTR as a cAMP-dependent regulator of sodium channels

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

The amiloride-inhibitable Na+ conductance is reduced by the cystic fibrosis transmembrane conductance regulator in normal but not in cystic fibrosis airways.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Cystic fibrosis transmembrane conductance regulator inverts protein kinase A-mediated regulation of epithelial sodium channel single channel kinetics

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Hyperabsorption of Na+ and raised Ca(2+)-mediated Cl- secretion in nasal epithelia of CF mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in betaENaC-overexpressing mice.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Acute and long-term amiloride inhalation in cystic fibrosis lung disease. A rational approach to cystic fibrosis therapy

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Nebulised amiloride in respiratory exacerbations of cystic fibrosis: a randomised controlled trial

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

French multicenter randomized double-blind placebo-controlled trial on nebulized amiloride in cystic fibrosis patients. The Amiloride-AFLM Collaborative Study Group

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Role of cilia, mucus, and airway surface liquid in mucociliary dysfunction: lessons from mouse models

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Altering airway surface liquid volume: inhalation therapy with amiloride and hyperosmotic agents

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Pharmacological Properties of N-(3,5-Diamino-6-chloropyrazine-2-carbonyl)-N'-4-[4-(2,3-dihydroxypropoxy)phenyl]butyl-guanidine Methanesulfonate (552-02), a Novel Epithelial Sodium Channel Blocker with Potential Clinical Efficacy for Cystic Fibrosis L

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

The guinea-pig tracheal potential difference as an in vivo model for the study of epithelial sodium channel function in the airways

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Mucus clearance and lung function in cystic fibrosis with hypertonic saline

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Regulation of the epithelial Na+ channel and airway surface liquid volume by serine proteases

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Na+ transport in normal and CF human bronchial epithelial cells is inhibited by BAY 39-9437.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Regulation of the epithelial sodium channel by serine proteases in human airways

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Camostat attenuates airway epithelial sodium channel function in vivo through the inhibition of a channel-activating protease

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Killing the messenger: short RNAs that silence gene expression

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Epithelial sodium channel inhibition in primary human bronchial epithelia by transfected siRNA.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

RNA interference for alpha-ENaC inhibits rat lung fluid absorption in vivo

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

RNA interference for CFTR attenuates lung fluid absorption at birth in rats

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Inefficient cationic lipid-mediated siRNA and antisense oligonucleotide transfer to airway epithelial cells in vivo.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Regulation of transepithelial ion transport and intracellular calcium by extracellular ATP in human normal and cystic fibrosis airway epithelium

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Inhibition of amiloride-sensitive epithelial Na(+) absorption by extracellular nucleotides in human normal and cystic fibrosis airways

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

The CF salt controversy: in vivo observations and therapeutic approaches.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Denufosol: a review of studies with inhaled P2Y(2) agonists that led to Phase 3.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

TMEM16A, a membrane protein associated with calcium-dependent chloride channel activity

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Expression cloning of TMEM16A as a calcium-activated chloride channel subunit

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

TMEM16A confers receptor-activated calcium-dependent chloride conductance

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

TMEM16A inhibitors reveal TMEM16A as a minor component of calcium-activated chloride channel conductance in airway and intestinal epithelial cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Cystic fibrosis transmembrane conductance regulator modulators for personalized drug treatment of cystic fibrosis: progress to date.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Understanding how cystic fibrosis mutations cause a loss of Cl- channel function.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Mouse models of cystic fibrosis: phenotypic analysis and research applications

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Chimeric constructs endow the human CFTR Cl- channel with the gating behavior of murine CFTR

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Cystic fibrosis across Europe: EuroCareCF analysis of demographic data from 35 countries

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Guideline on the design and conduct of cystic fibrosis clinical trials: the European Cystic Fibrosis Society-Clinical Trials Network (ECFS-CTN).

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Developing new products in cystic fibrosis: needs and obstacles for activities of small and middle-sized companies

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Calcium-pump inhibitors induce functional surface expression of Delta F508-CFTR protein in cystic fibrosis epithelial cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Rescue of DeltaF508-CFTR (cystic fibrosis transmembrane conductance regulator) by curcumin: involvement of the keratin 18 network

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Maintaining low Ca2+ level in the endoplasmic reticulum restores abnormal endogenous F508del-CFTR trafficking in airway epithelial cells.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Partial correction of cystic fibrosis defects with PLGA nanoparticles encapsulating curcumin

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Selective inhibition of endoplasmic reticulum-associated degradation rescues DeltaF508-cystic fibrosis transmembrane regulator and suppresses interleukin-8 levels: therapeutic implications.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

High-content functional screen to identify proteins that correct F508del-CFTR function

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

A cystic fibrosis respiratory epithelial cell chronically treated by miglustat acquires a non-cystic fibrosis-like phenotype

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Anti-inflammatory effect of miglustat in bronchial epithelial cells.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Benzo(c)quinolizinium drugs inhibit degradation of Delta F508-CFTR cytoplasmic domain.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

MPB-07 Reduces the Inflammatory Response toPseudomonas aeruginosain Cystic Fibrosis Bronchial Cells

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Sildenafil (Viagra) corrects DeltaF508-CFTR location in nasal epithelial cells from patients with cystic fibrosis.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Pharmacological modulation of cGMP levels by phosphodiesterase 5 inhibitors as a therapeutic strategy for treatment of respiratory pathology in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Structural analog of sildenafil identified as a novel corrector of the F508del-CFTR trafficking defect

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Potent s-cis-locked bithiazole correctors of DeltaF508 cystic fibrosis transmembrane conductance regulator cellular processing for cystic fibrosis therapy

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Chemical conjugation of DeltaF508-CFTR corrector deoxyspergualin to transporter human serum albumin enhances its ability to rescue Cl- channel functions.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Evidence of CFTR function in cystic fibrosis after systemic administration of 4-phenylbutyrate

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-diallyl-8-cyclohexylxanthine (DAX).

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Activation of wild-type and deltaF508-CFTR by phosphodiesterase inhibitors through cAMP-dependent and -independent mechanisms

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Direct action of genistein on CFTR.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Genistein activates CFTR Cl- channels via a tyrosine kinase- and protein phosphatase-independent mechanism

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Defective function of the cystic fibrosis-causing missense mutation G551D is recovered by genistein

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Correction of G551D-CFTR transport defect in epithelial monolayers by genistein but not by CPX or MPB-07.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Two mechanisms of genistein inhibition of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in murine cell line

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

The cystic fibrosis mutation G551D alters the non-Michaelis-Menten behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) channel and abolishes the inhibitory Genistein binding site.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Dietary genistein stimulates anion secretion across female murine intestine.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Structural determinants for activation and block of CFTR-mediated chloride currents by apigenin

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Development of substituted Benzo[c]quinolizinium compounds as novel activators of the cystic fibrosis chloride channel.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Synthesis, SAR, crystal structure, and biological evaluation of benzoquinoliziniums as activators of wild-type and mutant cystic fibrosis transmembrane conductance regulator channels.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

3-(2-Benzyloxyphenyl)isoxazoles and isoxazolines: synthesis and evaluation as CFTR activators

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

CFTR activation in human bronchial epithelial cells by novel benzoflavone and benzimidazolone compounds

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Benzoflavone activators of the cystic fibrosis transmembrane conductance regulator: towards a pharmacophore model for the nucleotide-binding domain

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

High-affinity activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Potent stimulation and inhibition of the CFTR Cl(-) current by phloxine B.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Phloxine B interacts with the cystic fibrosis transmembrane conductance regulator at multiple sites to modulate channel activity

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

The glycine residues G551 and G1349 within the ATP-binding cassette signature motifs play critical roles in the activation and inhibition of cystic fibrosis transmembrane conductance regulator channels by phloxine B.

1 reference

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7 January 2021

Differential sensitivity of the cystic fibrosis (CF)-associated mutants G551D and G1349D to potentiators of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Nanomolar affinity small molecule correctors of defective Delta F508-CFTR chloride channel gating.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Curcumin stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Phenylglycine and sulfonamide correctors of defective delta F508 and G551D cystic fibrosis transmembrane conductance regulator chloride-channel gating

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Mutation-specific potency and efficacy of cystic fibrosis transmembrane conductance regulator chloride channel potentiators.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Activating cystic fibrosis transmembrane conductance regulator channels with pore blocker analogs

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Antihypertensive 1,4-dihydropyridines as correctors of the cystic fibrosis transmembrane conductance regulator channel gating defect caused by cystic fibrosis mutations.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Structure-activity relationship of 1,4-dihydropyridines as potentiators of the cystic fibrosis transmembrane conductance regulator chloride channel

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

4-Chlorobenzo[F]isoquinoline (CBIQ), a novel activator of CFTR and DeltaF508 CFTR.

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

Discovery of pyrrolo[2,3-b]pyrazines derivatives as submicromolar affinity activators of wild type, G551D, and F508del cystic fibrosis transmembrane conductance regulator chloride channels

1 reference

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7 January 2021

Sulfamoyl-4-oxoquinoline-3-carboxamides: novel potentiators of defective DeltaF508-cystic fibrosis transmembrane conductance regulator chloride channel gating

1 reference

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7 January 2021

Identification of a novel water-soluble activator of wild-type and F508del CFTR: GPact-11a

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

A phase I randomized, multicenter trial of CPX in adult subjects with mild cystic fibrosis

1 reference

https://api.crossref.org/works/10.1016%2FS1569-1993%2811%2960018-0

7 January 2021

10.1016/S1569-1993(11)60018-0

Identifiers

DOI

1 reference

24 August 2017

1 reference